POS0884 DESCRIPTIVE EPIDEMIOLOGY OF SEVERE CARDIAC INVOLVEMENT IN SYSTEMIC SCLEROSIS: A BICENTRIC RETROSPECTIVE STUDY ON 459 PATIENTS
Authors: E. Bories, S. DE Almeida Chaves, T. Porel, L. Alric, L. Astudillo, F. Gaches, M. Michaud, F. Catros, G. Prevot, L. Sailler, D. Adoue, O. Lairez, G. Pugnet
Journal: Annals of the Rheumatic Diseases (2022)

Background The prevalence of cardiac involvement in systemic sclerosis (SSc) varies in the literature between 3% and 44% and represents a leading cause of mortality in this disease. The incidence of severe cardiac involvement and the factors associated with the occurrence of severe cardiac involvement are not known in the literature. Objectives The objective of this study was to evaluate the incidence, prognosis and factors associated with the occurrence of severe cardiac involvement during SSc course. Methods We conducted a retrospective, bi-centric study from January 1, 1966 to December 31, 2018. The patients included had a diagnosis of SS according to the ACR/EULAR 2013 criteria. The primary endpoint was the occurrence of severe cardiac involvement. Cardiac involvement was defined by the presence of at least one of the following elements: death of cardiovascular origin, left ventricular ejection fraction less than or equal to 50%, abnormality of at least 3 measurement parameters of diastolic function, global longitudinal strain less than or equal to 18 in absolute value, ventricular tachycardia, ventricular extrasystoles requiring intervention or elevated troponin. Patients with associated myositis and whose only criterion for cardiac involvement was elevated troponin were not included in the group with cardiac involvement. Severe cardiac involvement was defined by the occurrence of hospitalization for cardiovascular reasons or by death of cardiovascular origin. Univariable and multivariable Cox proportional hazards models were used to determine variables associated with severe cardiac involvement occurrence. Survival analysis was performed using the Kaplan-Meier method with comparisons performed using the log rank test. Results Four hundred and fifty-nine patients with SSc were included and were followed for a median of 7.1 years [3.1; 13.3]. The median age of our population was 54 years old. There were 81% of women, 77% of patients had limited cutaneous SSc, 15% diffuse cutaneous SSc and 8% SSc sine scleroderma. Of the 459 patients, 105 (23%) had cardiac involvement and 56 (12%) severe cardiac involvement. The incidence of severe cardiac involvement was 2.42 per 100 patient years. Ninety-six hospitalizations were recorded, including 40 (42%) for acute heart failure, 19 (20%) for arrhythmia, 5 (5%) for acute pericarditis, 6 (6%) for acute myocarditis and 14 (15 %) for coronary artery disease (acute coronary syndrome, myocardial infarction or coronary revascularization). The independent factors associated with severe cardiac involvement in SSc were age over 54 years at SSc-diagnosis (OR = 3.21 95% CI [1.73; 5.95], p &lt; 0.001), the presence of myositis (OR = 5.01 95% CI [1.89; 13.28], p = 0.001), pericardial involvement (OR = 3.79 95% CI [2.05; 7.03]; p &lt; 0.001) or scleroderma renal crisis (OR = 4.72 95% CI [2.05; 10.92], p &lt; 0.001). The survival rate of patients with severe cardiac involvement was 70% at 5 years and 53% at 10 years. Patients with severe cardiac involvement had a mortality risk three times greater than patients without severe cardiac involvement, HR = 3.1 (95% CI [1.7; 5.7], p&lt;0.0001) (Figure 1). Pericardial involvement was an independent risk factor for mortality, HR = 2.0 (95% CI [1.02; 4.0], p=0.04). Figure 1. Survival of patients with severe cardiac involvement of systemic scleroderma. HR: Hazard ratio; 95% CI: 95% Confidence interval; Nb at risk: Number at risk Conclusion We need to focus our clinical attention on diagnosing and manage cardiac involvement in SSc, as severe cardiac involvement is not uncommon and is responsible for a poor prognosis. Disclosure of Interests None declared